Esophageal Atresia - Arnold Coran: Update Course 2014

Cameron Haight performed the first esophageal atresia repair in 1941 and managed over 300 cases during his career.

The incidence of proximal fistula in type C esophageal atresia (with distal fistula) is approximately 10% based on a Netherlands study, significantly higher than the previously reported 1%.

The incidence of proximal fistula in pure esophageal atresia is much higher than in type C esophageal atresia.

A pouchogram can help determine the side of the aortic arch when timely cardiology evaluation is not available.

Contrast pouchography using approximately 1.5 cc of water-soluble contrast carries low aspiration risk and can identify proximal fistulas or suggest their presence when the upper pouch appears narrower than expected.

Air insufflation into the proximal pouch on plain radiograph can help identify proximal fistulas; absence of a dilated proximal pouch raises concern for a proximal fistula.

Gastrostomy tube placement should be performed in the first 24 hours for pure esophageal atresia cases.

Blind advancement of Bakes dilators from the gastrostomy site can push the gastric fundus up into the left chest, falsely suggesting a shorter gap than actually exists.

Neonatal gastroscope visualization of the distal esophageal segment under fluoroscopy is the best way to assess gap distance.

The gastroesophageal junction in neonates is remarkably small, making blind instrumentation difficult.

Contrast studies do not show the full extent of the lower esophageal segment.

Initial gap assessment should be delayed at least three weeks after gastrostomy placement to allow the gastrostomy site to mature and avoid displacing the stomach during endoscopy.

A gap of three vertebral bodies or less is reasonable to attempt primary repair, with the likelihood of success depending partly on the size of the upper pouch.

There is no need to rush primary repair in the early neonatal period at three weeks; waiting 12 weeks is reasonable as babies grow well with gastrostomy feedings.

The Foker procedure is associated with complications including massive gastroesophageal reflux, aspiration, and potentially poor long-term esophageal motility and function.

Traditional teaching suggests esophageal replacement should not be performed until the child can sit upright or walk (between six and 12 months) to minimize reflux complications.

Esophageal replacement options include colon interposition, gastric pull-up, jejunal interposition, and reverse gastric tube.

At approximately three months, the gap should be reassessed and a decision made, as most believe the gap will not grow significantly larger after this time.

Sequential gap studies should be performed every three weeks after the initial assessment; if no change is observed, waiting longer is not worthwhile.

Reverse gastric tube without spit fistula can be performed between three and six months of age for long-gap esophageal atresia.

If esophageal replacement is delayed beyond six months, a spit fistula should probably be created because the child will never eat without it.